December 3 month appointment

This is a hard post to write, and Jason asked me to write it. We knew that things had declined since the September appointment. We brought a list of questions and things we anticipated needing before our next appointment. The standard of care is 3 month appointments. We knew lung function had decreased and weight had declined because Jason tires easily and we can see weight loss. We also did not plan for SUCH a long day. This appointment was an interdisciplinary one that included seeing a doctor, several nurses, nutritionist, students from Georgia Tech, research, physical therapy, etc. We saw so many people and were at that appointment for more than 6 hours. Thank you to the volunteers that provided snacks!

Nutrition: Jason has lost more than 25 pounds in 3 months. We've tried at home with fruit smoothies and nutritional supplements of Cellect, but just have not gotten enough in. The nutritionist gave pointers and we are adding protein ice cream milk shakes (hello 30 grams of protein and 700 calories per shake), snacks several times a day, and ensure. Cellect is something Jason started back in October after his Uncle Charlie recommended it. This product is a dietary supplement that includes gelatin, cod liver oil, vitamins, 74 trace minerals, milk thistle seed extract, etc. It is thought to help maintain the body’s normal functions, to boost the immune system, and to help fight diseases by fixing the body’s chemical imbalances. They also did a quick swallow study in clinic because they noticed that Jason swallowed twice sometimes when we only swallow once. An official swallow study was completed early January under imaging and showed slight aspiration (instead of food going to stomach, it goes towards the lungs) that Jason was able to clear himself by clearing his throat.

Lungs: Jason's lung function has declined to 1/2 (of a "normal" person his size) in 3 months. They test strength of the diaphragm and capacity of the lungs. Strength was the significant decrease. Remember that ALS is the dysfunction of nerves that control muscles. The diaphragm is the primary muscle used in the process of inhalation. It lies at the base of the chest and separates the abdominal cavity from the chest/thoracic cavity. It was a surprise that the decrease was by this much. However, we did walk all the way to the clinic. Emory clinics are very large, and I truly believe if we had use a wheelchair these numbers would have been better. Within 2 weeks of the appointment, a respiratory therapist came to the house and set up a cough assist machine (top) and Trilogy machine to sleep with at night (bottom). Jason's ability to cough is declined and weak. It is important to clear out the lungs regularly to prevent pneumonia. This machine pushes air as Jason takes a deep breath, then pulls it out inducing a cough. Recommendations are to do 4 sets of 5 at least twice a day. The Trilogy machine is very similar to CPAP, which people who have sleep apnea sleep with. Settings can be changed as his lungs change. There is a round grey thing in front-that holds a canister to humidify the air.

Mobility: Jason wears a brace that prevents foot drop on both feet, but it is just not enough. We planned to ask for a prescription for a walker, but the ALS association representative said they had one in the "borrow" closet. She brought it to the house within 3 days and you can see the extra stuff I added to the right hand-it just added bulk to help Jason use it. Also, the physical therapists recommended getting "fitted" for a power wheelchair. This process of getting approved by insurance and building the wheelchair can take 90 days. The measuring and going through options took some time. We expect that to be delivered to the house in March.

Research: We were able to discuss in detail with Dr. Fournier the options. I am very interested in the stem cell trials. This is probably because I have bone marrow transplant experience with my nursing career. Once you take a stem cell trials, you cannot really do further trials because stem cells live so long in the body, so we want to wait to do this (plus, they have just completed stage 2 of the trials, so technically aren't open now). There is a pending pill, endaravone, that we are waiting to be approved in the US hopefully in June 2017. Dr. Fournier says that they will have a pill trial in February opening as well. Other trials are information gathering trials (we are doing all of these) and ones that you have to stay in the hospital for a week at a time. We did learn that with Emory trials, there is a blind placebo group. We are NOT guaranteed to get any drug at all, even if we sign up for a trial. There are some ethical issues here, but that is how research goes. We also learned that Jason can continue the only FDA drug approved for ALS, rilutek, drug any trial that we do sign up for in the future.

Other: Students at Georgia Tech came into the room to discuss robotics being done. It would require extra trips into Atlanta to participate, so we will see. We did get a form to take to the DMV for handicapped parking. We did get a prescription for something similar to waffle boots to wear at night to help with foot drop.